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A familial syndrome of infantile optic atrophy, movement disorder, and spastic paraplegia

New pediatric Unit, Loewenabin Hospital Rehabilitation Center, Ra'anana, Tel-Aviv, Israel (Drs. Coateff and Apter)
Department of Neurology, Beilinson Medical Center, Petah-Tikva, Tel-Aviv, Israel (Dr. Cadoth)
Department of Ophthalmology, Colda Medical Center, Petah-Tikva, Tel-Aviv, Israel (Drs. Prinlnic and Savir)
Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel (Drs. Costeff, Cadoth, Apter, and Savir).

We describe 19 cases of a familial syndrome consisting of infantile optic atrophy and an early movement disorder in which chorea predominated. About one-half the patients developed spastic paraphrases during the second decade of life. Ataxia and cognitive deficits were common, usually of mild degree. Seventeen of the patients were females. Sixteen had similarly affected siblings, but none had affected parents. All but one belonged to the Iraqi Jewish community in Israel, giving a minimal prevalence rate in this ethnic group of about 1:10,000.

Address correspondence and reprint requests to Dr. Coateff, Laewenstein Hospital Rehabilitation Center, P.O.B. 3. Ra'ahana 43100, Israel.

Received June 28,1988. Accepted for publication in final form October 24, 1988.

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